WorksheetsHEMA-12
Total questions: 110
Worksheet time: 55mins
Name
Class
Date
1.
What enzyme is deficient in Niemann-Pick disease?
a)
a. β-glucocerebrosidase
b)
b. Sphingomyelinase
c)
c. Hexosaminidase A
d)
d. Galactocerebrosidase
2.
Niemann-Pick disease follows what type of inheritance?
a)
a. Autosomal recessive
b)
b. Autosomal dominant
c)
c. X-linked recessive
d)
d. X-linked dominant
3.
Niemann-Pick disease is most common among which population?
a)
a. African Americans
b)
b. Ashkenazi Jews
c)
c. Mediterranean descent
d)
d. Asians
4.
Which organs are greatly enlarged in Niemann-Pick disease?
a)
a. Heart and lungs
b)
b. Liver and spleen
c)
c. Brain and kidneys
d)
d. Pancreas and gallbladder
5.
What is the characteristic cell seen in Niemann-Pick disease?
a)
a. Gaucher cell
b)
b. Pick’s cell
c)
c. Reed-Sternberg cell
d)
d. Auer body
6.
What is the cytoplasmic appearance of a Pick’s cell?
a)
a. Crumpled tissue paper appearance
b)
b. Swollen with many small lipid droplets
c)
c. Fine basophilic granules
d)
d. Clear vacuolated cytoplasm
7.
Niemann-Pick disease is usually fatal by what age?
a)
a. 1 year
b)
b. 3 years
c)
c. 5 years
d)
d. 10 years
8.
What enzyme is deficient in Gaucher disease?
a)
a. β-glucocerebrosidase
b)
b. Sphingomyelinase
c)
c. Hexosaminidase A
d)
d. Arylsulfatase A
9.
What is the substance that accumulates in Gaucher disease?
a)
a. Sphingomyelin
b)
b. Glucocerebrosides
c)
c. Gangliosides
d)
d. Ceramides
10.
What is the typical cytoplasmic appearance of a Gaucher cell?
a)
a. Foamy
b)
b. Cystic
c)
c. Wrinked
d)
d. Dirty
11.
Gaucher cell appearance
a)
Onion-like skin or crumpled tissue paper appearance
b)
Garlic-like skin
c)
Cigar-shaped
d)
Witchbroom stick-like appearance
12.
Where are Gaucher cells primarily found?
a)
a. Blood
b)
b. Bone marrow
c)
c. Lymph nodes
d)
d. Spleen
13.
What is the shape and position of the nucleus in Gaucher cells?
a)
a. Large and central
b)
b. Small and eccentric
c)
c. Lobulated and basophilic
d)
d. Binucleated
14.
Gaucher disease follows what inheritance pattern?
a)
a. X-linked recessive
b)
b. X-linked dominant
c)
c. Autosomal recessive
d)
c. Autosomal dominant
15.
What type of inheritance does Chediak-Higashi syndrome have?
a)
a. X-linked recessive
b)
b. X-linked dominant
c)
c. Autosomal recessive
d)
c. Autosomal dominant
16.
Chediak-Higashi syndrome primarily affects what cellular function?
a)
a. DNA replication
b)
b. Lysosomal trafficking and fusion
c)
c. Protein synthesis
d)
d. RNA transcription
17.
Which cells contain large, abnormal cytoplasmic granules in Chediak-Higashi syndrome?
a)
a. Erythrocytes
b)
b. Phagocytes (granulocytes and monocytes)
c)
c. Platelets
d)
d. Megakaryocytes
18.
The abnormal granules in phagocytes in Chediak-Higashi syndrome are:
a)
a. Peroxidase positive
b)
b. Peroxidase negative
c)
c. PAS negative
d)
d. Sudan black negative
19.
The abnormal granules in lymphocytes in Chediak-Higashi syndrome are:
a)
a. Peroxidase positive
b)
b. Peroxidase negative
c)
c. PAS negative
d)
d. Sudan black negative
20.
Partial albinism in Chediak-Higashi syndrome is due to:
a)
a. Defective melanin synthesis
b)
b. Abnormal melanosome packaging
c)
c. Tyrosinase deficiency
d)
d. Autoimmune destruction of melanocytes
21.
Which of the following is a characteristic feature of Chediak-Higashi syndrome?
a)
a. Hyperpigmentation, reddish hair, lymphoma
b)
b. Silvery hair, pale skin and photophobia
c)
c. Silvery hair, dark skin and photophobia
d)
d. Silvery hair, microcephaly, and photophobia
22.
What is the inheritance pattern of Wiskott-Aldrich syndrome?
a)
a. Autosomal recessive
b)
b. X-linked recessive
c)
c. X-linked dominant
d)
d. Autosomal dominant
23.
What is the classic triad of Wiskott-Aldrich syndrome?
a)
a. Thrombocytopenia, eczema, immunodeficiency
b)
b. Thrombocytopenia, eczema, immunocompetency
c)
c. Thrombocytosis, eczema, immunodeficiency
d)
d. Thrombocytosis, eczema, immunocompetency
24.
What type of platelet abnormality is seen in Wiskott-Aldrich syndrome?
a)
a. Giant platelets
b)
b. Small platelets
c)
c. Absent granules
d)
d. Platelet hyperactivity
25.
Wiskott-Aldrich thrombocytes show:
a)
a. Increased dense granules
b)
b. Decreased dense granules
c)
c. Normal dense granules
d)
d. No change in granules
26.
Which immune cells are dysfunctional in Wiskott-Aldrich syndrome?
a)
a. B cells only
b)
b. B cells and T cells
c)
c. B cells, T cells, NK cells, Macrophage, neutrophils and monocytes
d)
d. B cells, T cells, NK cells, neutrophils, and monocytes
27.
Wiskott-Aldrich syndrome increases susceptibility to which infections?
a)
a. Bacterial
b)
b. Bacterial and viral
c)
c. Bacterial, viral, and fungal
d)
d. Bacterial, viral, fungal, and parasitic
28.
Pseudo-Gaucher cells may appear in all of the following except:
a)
a. Thalassemia
b)
b. Chronic myeloid leukemia
c)
c. Diabetes mellitus
d)
d. Non-Hodgkin lymphoma
e)
e. Plasma cell neoplasm
29.
May-Hegglin anomaly follows what type of inheritance?
a)
a. Autosomal dominant
b)
b. Autosomal recessive
c)
c. X-linked recessive
d)
d. X-linked dominant
30.
Which of the following inclusions are characteristic of May-Hegglin anomaly?
a)
a. Auer rods
b)
b. Döhle body-like inclusions
c)
c. Howell-Jolly bodies
d)
d. Pappenheimer bodies
31.
The inclusions seen in May-Hegglin anomaly are typically:
a)
a. Red and rod-shaped
b)
b. Gray-blue and spindle-shaped
c)
c. Blue-green and round-shaped
d)
d. Purple and circular
32.
What hematologic abnormalities are often seen in May-Hegglin anomaly?
a)
a. Leukocytosis, thrombocytosis, and small platelets
b)
b. Leukopenia, thrombocytopenia, and giant platelets
c)
c. Leukopenia, thrombocytosis, and giant platelets
d)
d. Leukopenia, thrombocytopenia, and small platelets
33.
What is the hallmark feature of Pelger-Huët anomaly?
a)
a. Failure of the neutrophil nucleus to segment
b)
b. Hypersegmented neutrophils
c)
c. Large azurophilic granules
d)
d. Cytoplasmic vacuolization
34.
Pelger-Huët anomaly is inherited as:
a)
a. Autosomal recessive
b)
b. Autosomal dominant
c)
c. X-linked recessive
d)
d. X-linked dominant
35.
What term is used to describe the characteristic nuclear shape in Pelger-Huët anomaly?
a)
a. Drumstick form
b)
b. Pince-nez or spectacle form
c)
c. Ring form or round form
d)
d. Horseshoe form
36.
Pelger-Huët anomaly is caused by a mutation in which gene?
a)
a. MPO gene
b)
b. Lamin β-receptor gene
c)
c. BCR-ABL gene
d)
d. JAK2 gene
37.
The lamin β-receptor protein is primarily involved in:
a)
a. Cell surface antigen recognition
b)
b. Leukocyte nuclear shape changes during maturation
c)
c. RNA transport
d)
d. Mitochondrial metabolism
38.
In Homozygous Pelger-Huët anomaly, all neutrophils typically have:
a)
a. Bilobed nuclei
b)
b. Round nuclei
c)
c. Trilobed nuclei
d)
d. Pyknotic nuclei
39.
In Heterozygous Pelger-Huët anomaly, approximately what percentage of neutrophils are affected?
a)
a. 5–10%
b)
b. 25–40%
c)
c. 55–93%
d)
d. 100%
40.
What are the typical nuclear shapes observed in unilobed Pelger-Huët anomaly?
a)
a. Round, ovoid, or peanut-shaped
b)
b. Triangular or lobulated
c)
c. Crescent-shaped
d)
d. Irregular and multilobed
e)
e. Round, flat, or japanese-lantern shaped
41.
In true Pelger-Huët anomaly, neutrophil function is:
a)
a. Absent
b)
b. Decreased
c)
c. Normal
d)
d. Hyperactive
42.
The acquired form of Pelger-Huët anomaly is also known as:
a)
a. Pseudo–Pelger-Huët anomaly
b)
b. Hyper-Pelger-Huët anomaly
c)
c. Myeloid Pelger-Huët anomaly
d)
d. Secondary Pelger-Huët anomaly
43.
Pseudo–Pelger-Huët cells are commonly seen in all of the following EXCEPT:
a)
a. Acute myeloid leukemia
b)
b. Myelodysplastic syndromes
c)
c. HIV infection
d)
d. Iron deficiency anemia
e)
e. Chronic myeloproliferative neoplasms
44.
How do neutrophils in true Pelger-Huët anomaly differ from pseudo–Pelger-Huët cells?
a)
a. True PHA shows normal granulation
b)
b. Pseudo-PHA shows normal granulation
c)
c. True PHA shows hypogranularity
d)
d. True PHA shows abnormal granulation
e)
e. None of the above
45.
A neutrophil is considered hypersegmented if its nucleus has how many lobes or more?
a)
3
b)
4
c)
5
d)
6
46.
Hypersegmented neutrophils are most commonly associated with which condition?
a)
a. Iron deficiency anemia
b)
b. Megaloblastic anemia
c)
c. Aplastic anemia
d)
d. Hemolytic anemia
47.
Which of the following disorders may also show hypersegmented neutrophils?
a)
a. Myelodysplastic syndrome
b)
b. Leukemoid reaction
c)
c. Infectious mononucleosis
d)
d. Chediak-Higashi syndrome
48.
Myelokathexis is characterized by normal production but:
a)
a. Impaired release of granulocytes into circulation
b)
b. Impaired release of agranulocytes into circulation
c)
c. Increased apoptosis of RBCs
d)
d. Increased marrow fibrosis
49.
Aside from hypermature neutrophils, what is a characteristic morphologic feature of neutrophils in myelokathexis?
a)
a. Round nuclei with fine chromatin
b)
b. Hypersegmentation and hypercondensed chromatin
c)
c. Hyposegmentation and hypercondensed chromatin
d)
d. Hypersegmentation and hypocondensed chromatin
50.
The LE cell is usually which type of cell that has phagocytosed nuclear material?
a)
a. Monocyte
b)
b. Neutrophil
c)
c. Eosinophil
d)
d. Basophil
51.
What material is engulfed by the neutrophil to form an LE cell?
a)
a. Antibody-coated nucleus of another neutrophil
b)
b. Mitochondrial debris
c)
c. RBC fragments
d)
d. Platelet granules
52.
The ingested material in LE cells appears as a:
a)
a. Fine granular mass
b)
b. Homogenous globular nuclear body
c)
c. Eosinophilic inclusion
d)
d. Clear vacuole
53.
The ingested material in LE cells appears as a:
a)
a. Fine granular mass
b)
b. Homogenous globular nuclear body
c)
c. Eosinophilic inclusion
d)
d. Clear vacuole
54.
LE cells are most commonly associated with:
a)
a. Rheumatoid arthritis
b)
b. Systemic lupus erythematosus (SLE)
c)
c. Chediak-Higashi syndrome
d)
d. Chronic myeloid leukemia
55.
LE cells may also be seen in:
a)
a. Connective tissue disorders
b)
b. Hemolytic anemias
c)
c. Iron deficiency anemia
d)
d. Myeloproliferative neoplasms
56.
The formation of LE cells is primarily:
a)
a. An in vivo phenomenon
b)
b. An in vitro phenomenon
c)
c. A tissue-level reaction only
d)
d. A bone marrow–specific event
57.
= round, ovoid, or peanut shaped
a)
Unilobed
b)
Bilobed
c)
Homozygous PHA
d)
Heterozygous PHA
58.
pectacle-like (“pince nez”) morphology with nuclei attached by a thin filament
a)
Unilobed
b)
Bilobed
c)
Homozygous PHA
d)
Heterozygous PHA
59.
= all neutrophils are affected and demonstrate round nuclei
a)
Unilobed
b)
Bilobed
c)
Homozygous PHA
d)
Heterozygous PHA
60.
= 55% to 93% of the neutrophil population are affected (there is generally a mixture of all of the aforementioned nuclear shapes)
a)
Unilobed
b)
Bilobed
c)
Homozygous PHA
d)
Heterozygous PHA
61.
(aka: Morula cell or Mott cell)
a)
Tart cell
b)
Reider cell
c)
Grape cell
d)
Hairy cell
62.
A monocyte that has ingested a whole lymphocyte or a nucleus with an identifiable nuclear chromatin is known as a:
a)
a. LE cell
b)
b. Tart cell
c)
c. Rieder cell
d)
d. Grape cell
63.
Tart cells may be seen in which of the following conditions?
a)
a. Drug sensitivity
b)
b. Systemic lupus erythematosus
c)
c. Chronic lymphocytic leukemia
d)
d. Multiple myeloma
64.
Aside from being artificially formed through blood smear preparation, Rieder cells are often observed in which hematologic disorder?
a)
a. Hairy cell leukemia
b)
b. Chronic lymphocytic leukemia
c)
c. Multiple myeloma
d)
d. Acute myelogenous leukemia
65.
A lymphocyte with a notched, lobulated, or cloverleaf-like nucleus is characteristic of:
a)
a. Rieder cell
b)
b. Pelger-Huet cell
c)
c. Tart cell
d)
d. Hairy cell
66.
Which of the following cells is described as an abnormal plasma cell with cytoplasm completely filled with Russell bodies?
a)
a. Tart cell
b)
b. Hairy cell
c)
c. Grape cell
d)
d. Rieder cell
67.
The acronym CRAB (Hypercalcemia, Renal insufficiency, Anemia, Bone lesions) is associated with:
a)
a. Tart cell
b)
b. Hairy cell
c)
c. Grape cell
d)
d. Rieder cell
68.
-Found in Multiple Myeloma (Plasma Cell Myeloma)
a)
a. Tart cell
b)
b. Hairy cell
c)
c. Grape cell
d)
d. Rieder cell
69.
The acronym CRAB (Hypercalcemia, Renal insufficiency, Anemia, Bone lesions) is associated with:
a)
a. Hairy cell leukemia
b)
b. Pelger-Huet anomaly
c)
c. Plasma cell myeloma
d)
d. Infectious mononucleosis
70.
Hairy cells are best identified by their positive reaction to which enzyme?
a)
a. Peroxidase
b)
b. Tartrate-resistant acid phosphatase (TRAP)
c)
c. Myeloperoxidase (MPO)
d)
d. Lysozyme
71.
The cytoplasmic appearance of a Hairy cell is described as:
a)
a. Filled with Russell bodies
b)
b. Containing little cytoplasmic projections
c)
c. Having azurophilic granules
d)
d. Vacuolated and foamy
72.
Which isoenzyme of hairy cells is unique for being tartrate resistant?
a)
Isoenzyme 3
b)
Isoenzyme 5
c)
Isoenzyme 9
d)
Isoenzyme 2
73.
Isoenzyme 5 of acid phosphatase is unique for being:
a)
a. Inhibited by tartrate
b)
b. Produced by red blood cells
c)
c. Tartrate-resistant and abundant in Hairy cells
d)
d. Found only in platelets
74.
Grape cells, also known as Mott cells, are typically found in patients with:
a)
a. Plasma cell myeloma
b)
b. Chronic lymphocytic leukemia
c)
c. Hodgkin's lymphoma
d)
d. Myelodysplastic syndrome
75.
Presence of these cells is the definitive histologic characteristic of Hodgkin’s Disease
a)
Reed-Sternberg cell
b)
Flower cells
c)
Popcorn cells
d)
Sezary cells
76.
A large lymphoid cell which may demonstrate two nuclei (with eosinophilic nucleoli) and an abundant cytoplasm
a)
Reed-Sternberg cell
b)
Flower cells
c)
Popcorn cells
d)
Sezary cells
77.
Seen in Adult T Cell Leukemia
a)
Reed-Sternberg cell
b)
Flower cells
c)
Popcorn cells
d)
Sezary cells
78.
(aka: L and H cells) meaning, lymphocytic and histiocytic
a)
Reed-Sternberg cell
b)
Flower cells
c)
Popcorn cells
d)
Sezary cells
79.
Seen in NLPHL (Nodular Lymphocyte Predominant Hodgkin’s Lymphoma)
a)
Reed-Sternberg cell
b)
Flower cells
c)
Popcorn cells
d)
Sezary cells
80.
-Characterized by a cerebriform nucleus
a)
Reed-Sternberg cell
b)
Flower cells
c)
Popcorn cells
d)
Sezary cells
81.
Seen in Mycosis Fungoides (an example of a Non-Hodgkin’s Lymphoma)
a)
Reed-Sternberg cell
b)
Flower cells
c)
Popcorn cells
d)
Sezary cells
82.
Purple-red particles composed of precipitated mucopolysaccharides resembling very coarse toxic granules seen in neutrophils, eosinophils, and basophils are called:
a)
a. Döhle bodies
b)
b. Toxic granules
c)
c. Alder-Reilly inclusions
d)
d. Auer rods
83.
Most frequently seen in patients with Hunter, Hurler, and Maroteaux-Lamy
a)
a. Döhle bodies
b)
b. Toxic granules
c)
c. Alder-Reilly inclusions
d)
d. Auer rods
84.
Alder-Reilly inclusions are only occasionally seen in
a)
Neutrophils
b)
Eosinophils
c)
Basophils
d)
Monocytes
e)
Lymphocytes
85.
Toxic granulations are altered primary granules found in the cytoplasm of the neutrophils that appear as:
a)
a. Light blue cytoplasmic inclusions
b)
b. Dark-blue to black granules
c)
c. Purple-red particles
d)
d. Pink azurophilic granules
86.
Toxic granulations are found in which type of cell?
a)
a. Monocytes
b)
b. Lymphocytes
c)
c. Neutrophils
d)
d. Eosinophils
87.
Toxic granulations are most commonly seen in patients with:
a)
a. Lead poisoning and severe infections
b)
b. Chronic lymphocytic leukemia
c)
c. Pernicious anemia
d)
d. Infectious mononucleosis
88.
Linear or needle-like cytoplasmic inclusions formed from primary granules are known as:
a)
a. Döhle bodies
b)
b. Auer rods
c)
c. Alder-Reilly inclusions
d)
d. Toxic granulations
89.
Auer rods are characteristic of which hematologic malignancy?
a)
a. Acute myelogenous leukemia (AML)
b)
b. Chronic lymphocytic leukemia (CLL)
c)
c. Hodgkin lymphoma
d)
d. Multiple myeloma
90.
A leukemic cell containing bundles of Auer rods is called a:
a)
a. Faggot cell
b)
b. Mott cell
c)
c. Tart cell
d)
d. Rieder cell
91.
Round or oval blue-staining inclusions consisting of ribosomal RNA, seen in neutrophils during pregnancy, severe burns, aplastic anemia, scarlet fever and other infectious diseases, and following administration of toxic agents, are called:
a)
a. Döhle bodies
b)
b. Alder-Reilly inclusions
c)
c. Toxic granules
d)
d. Auer rods
92.
Döhle bodies are commonly found in which of the following conditions?
a)
a. Chronic myelogenous leukemia and Gaucher disease
b)
b. Pregnancy, severe burns, aplastic anemia, and scarlet fever
c)
c. Myelodysplastic syndrome and CLL
d)
d. Lead poisoning and sideroblastic anemia
93.
Döhle bodies are primarily composed of:
a)
a. Precipitated mucopolysaccharides
b)
b. Ribosomal RNA
c)
c. Denatured hemoglobin
d)
d. Lysosomal enzymes
94.
Alder-Reilly inclusions are composed of:
a)
a. Lipid droplets
b)
b. Precipitated mucopolysaccharides
c)
c. Denatured RNA
d)
d. Aggregated lysosomal enzymes
95.
Alder-Reilly inclusions are most frequently seen in which group of inherited disorders?
a)
a. Lipidoses
b)
b. Mucopolysaccharidoses
c)
c. Leukodystrophies
d)
d. Glycogen storage diseases
96.
What is the PAS (Periodic Acid-Schiff) reaction result for Döhle bodies?
a)
Negative
b)
Positive
c)
Variable
97.
What is the PAS (Periodic Acid-Schiff) reaction result for Döhle-body like inclusions in MHA?
a)
Negative
b)
Positive
c)
Variable
98.
Which of the following inclusions are smaller, round, and PAS-positive?
a)
a. Döhle body-like inclusions (May-Hegglin Anomaly)
b)
b. Döhle bodies
c)
c. Alder-Reilly inclusions
d)
d. Auer rods
99.
Döhle bodies are composed primarily of:
a)
a. Messenger RNA
b)
b. Ribosomal RNA
c)
c. Precipitated mucopolysaccharides
d)
d. Denatured DNA
100.
Döhle body-like inclusions in May-Hegglin Anomaly differ from typical Döhle bodies because they are:
a)
a. Smaller and round
b)
b. Larger and spindle-shaped
c)
c. Smaller and oval
d)
d. Irregular and granular
101.
The seen Dohle bodies of (MHA)May-Hegglin Anomaly are composed of:
a)
a. Ribosomal RNA
b)
b. Messenger RNA
c)
c. Precipitated RNA
d)
d. DNA fragments
102.
Which of the following inclusions are PAS-negative and composed of mRNA?
a)
a. Döhle bodies
b)
b. Alder-Reilly inclusions
c)
c. Döhle body-like inclusions in May-Hegglin Anomaly
d)
d. Toxic granulations
103.
Smaller
a)
Döhle bodies
b)
Döhle body-like inclusions in MHA
104.
PAS positive
a)
Döhle bodies
b)
Döhle body-like inclusions in MHA
105.
PAS Negative
a)
Döhle bodies
b)
Döhle body-like inclusions in MHA
106.
Contains messenger RNA
a)
Döhle bodies
b)
Döhle body-like inclusions in MHA
107.
Spindle shaped
a)
Döhle bodies
b)
Döhle body-like inclusions in MHA
108.
Larger
a)
Döhle bodies
b)
Döhle body-like inclusions in MHA
109.
contains rRNA
a)
Döhle bodies
b)
Döhle body-like inclusions in MHA
110.
Round
a)
Döhle bodies
b)
Döhle body-like inclusions in MHA
100 %
